Changing life trajectories for heart patients

Kathleen

When cardiologist Bradley Marino, MD, began practicing medicine 20 years ago, many children who underwent surgery for congenital heart disease (CHD) did not survive. Today, the survival rate for children who undergo cardiovascular surgery at Ann & Robert H. Lurie Children’s Hospital of Chicago is at 98 percent—the second highest rate of any pediatric hospital in the nation.

Now, Dr. Marino and his colleagues at the hospital’s Heart Center have a different challenge: maximizing the quality of life for the significant numbers of children with neurodevelopmental and psychosocial impairments due to brain injuries related to their congenital heart defects. The causes of these injuries include low oxygen levels, decreased brain blood flow and issues related to medical and surgical therapies.

 
Addressing developmental delays
Dr. Marino is an international leader in the relatively new field of cardiac neurodevelopmental outcomes. He is collaborating with neonatologist Raye-Ann DeRegnier, MD, to extend Lurie Children’s existing evaluation and support services for premature infants and survivors of complex congenital heart disease through 5 years of age. The new program, the Neo-Heart Developmental Support Program, will be one of few such programs in the U.S. It will add critically needed services for survivors of prematurity and congenital heart disease between 6 and 18 years of age. The program is supported by the Children’s Research Fund North Suburban Board.
 
"Because more children are surviving with congenital heart disease, we have a large population of patients with long-term developmental needs that must be addressed,” says Dr. Marino. “Many of these families have seen these behaviors throughout their child’s life, but have not been able to put a name to it or find help from specialists who understand the specific issues these children face.”
 
The program fills an essential need, as Lurie Children’s pediatric cardiovascular-thoracic surgery program is not only the largest in Illinois, but also one of the busiest in the nation. Last year, Heart Center surgeons performed 400 surgeries and 22 heart transplants.
 
Children with complex CHD often undergo multiple surgeries and long hospitalizations. Dr. Marino says that 75 percent of these patients are at risk for language impairment, Attention Deficit Hyperactivity Disorder, executive dysfunction, visual processing issues, fine and gross motor problems and behavioral and emotional difficulties. More than 30 percent of these children require remedial services in school including tutoring, special education, and physical, occupational and speech therapies.
 
The Neo-Heart Developmental Support Program helps families in several ways: surveillance (recognizing children who may be at risk for delays); screening (using standardized tools to identify and refine these risks); evaluation (identifying the specific impairments that affect a child); and management of these issues.
 
New heart, new challenges
Kathleen Conzemius is one of the first patients in the program. Although she is only 9, the third grader has undergone five open-heart surgeries at Lurie Children’s, including a heart transplant when she was 4. While her heart is healthy, Kathleen experiences cognitive delays that affect her progress in reading and math. During the school day, Kathleen works one-on-one with a resource teacher to prevent her from falling further behind her classmates.
 
“She is making strides, but it’s a slow process,” says Kathleen’s mom, Tegwen. “It’s so unfair, because she’s come so far and has beaten so many odds, but now she struggles with self-esteem issues.”
 
When Tegwen and her husband, Jim, first learned about the wealth of research on links between childhood heart surgery and developmental delays and deficits, she says it was as if a “light bulb clicked on.” When cardiology pediatric nurse practitioner Carrie Alden, a key member of the new support program, asked if they would be interested in enrolling, their response was immediate.
 
“We said, ‘absolutely!’” says Tegwen. “Kathleen’s developmental issues are really a hidden disability, because she looks and acts like any other kid her age. We’ve worked closely with her school, but know we need more help.”
 
Dr. Marino is also leading the development of five separate clinical centers of excellence or “bridge programs” in collaboration with Lurie Children’s Heart Center’s colleagues at Northwestern Memorial Hospital’s Bluhm Cardiovascular Institute. The goal is to create a continuum of care for patients with congenital heart disease from infancy to old age.
 
Research to save lifetimes
Dr. Marino also directs the Center for Cardiovascular Innovation at the hospital’s Stanley Manne Children’s Research Institute, and is focused on expanding research to help survivors of childhood congenital heart disease surgery. Current projects include a study on identifying predictors that either promote resilience or lessen depression in these patients, and another to develop interventions to address deficits in executive brain function and behavior and gross motor issues.
 
Clinical care and research are intertwined throughout all of Lurie Children’s programs, and philanthropy is essential for discoveries that will help children both today and in the future.
 
“One of the reasons I went into pediatric medicine was to not only save lives, but to save lifetimes,” says Dr. Marino. “With philanthropic support, we can intervene early to change a child’s trajectory in life.”

This article originally appeared in the Spring 2015 issue of Heroes.